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Information Patients and Family (5 links)
Childhood Ependymoma Treatment
National Cancer InstitutePDQ summaries are written and frequently updated by editorial boards of experts Further info.
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Collaborative Ependymoma Research Network
CERN Foundation
CERN Foundation is comprised of a dedicated group of scientists and adult and pediatric neuro-oncologists working to find new treatments for ependymoma.
Childhood Brain Tumor Foundation
Referenced article about childhood Ependymomas.
Information for Health Professionals / Researchers (4 links)
- PubMed search for publications about Brain, Ependymoma, Childhood - Limit search to: [Reviews]
PubMed Central search for free-access publications about Brain, Ependymoma, Childhood
MeSH term: EpendymomaUS National Library of Medicine
PubMed has over 22 million citations for biomedical literature from MEDLINE, life science journals, and online books. Constantly updated.
Childhood Ependymoma Treatment
National Cancer InstitutePDQ summaries are written and frequently updated by editorial boards of experts Further info.
Clinical Trials - Childhood Ependymoma
National Cancer Institute
Search of the NCI's database of 12,000+ clinical trials from around the world.
Collaborative Ependymoma Research Network
CERN Foundation
CERN Foundation is comprised of a dedicated group of scientists and adult and pediatric neuro-oncologists working to find new treatments for ependymoma.
Latest Research Publications
This list of publications is regularly updated (Source: PubMed).
Quantitative imaging analysis of posterior fossa ependymoma location in children.
Childs Nerv Syst. 2016; 32(8):1441-7 [PubMed] Article available free on PMC after 01/08/2017 Related Publications
METHODS: Pre-operative MRI examinations of the brain for 38 children with histopathologically proven posterior fossa ependymoma were analyzed. Tumor margin contours and anatomic landmarks were manually marked and used to calculate the centroid of each tumor. Landmarks were used to calculate a transformation to align, scale, and rotate each patient's image coordinates to a common coordinate space. Hierarchical cluster analysis of the location and morphological variables was performed to detect multivariate patterns in tumor characteristics. The ependymomas were also characterized as "central" or "lateral" based on published radiological criteria. Therapeutic details and demographic, recurrence, and survival information were obtained from medical records and analyzed with the tumor location and morphology to identify prognostic tumor characteristics.
RESULTS: Cluster analysis yielded two distinct tumor groups based on centroid location The cluster groups were associated with differences in PFS (p = .044), "central" vs. "lateral" radiological designation (p = .035), and marginally associated with multiple operative interventions (p = .064).
CONCLUSIONS: Posterior fossa ependymoma can be objectively classified based on quantitative analysis of tumor location, and these classifications are associated with prognostic and treatment factors.
Genomics and epigenetics: A study of ependymomas in pediatric patients.
Clin Neurol Neurosurg. 2016; 144:53-8 [PubMed] Related Publications
METHODS: CGH microarray, methylation and gene expression were performed through the Agilent platform. The results were analyzed with the software MatLab, MapViewer, DAVID, GeneCards and Hippie.
RESULTS: Amplification was found in 14q32.33, 2p22.3 and 8p22, and deletion was found in 8p11.23-p11.22 and 1q21.3. We observed 42.387 CpG islands with changes in their methylation pattern, in which we found 272 genes involved in signaling pathways related to carcinogenesis. We found 481 genes with altered expression. The genes IMMT, JHDMD1D, ASAH1, ZWINT, IPO7, GNAO1 and CISD3 were found to be altered among the three levels.
CONCLUSION: The 2p22.3, 8p11.23-p11.22 and 14q32.33 regions were identified as the most important; the changes in the methylation pattern related to cell cycle and cancer genes occurred in MIB2, FGF18 and ITIH5. The IPO7, GNAO1 and ASAH1 genes may play a major role in ependymoma development.
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SOX10 Distinguishes Pilocytic and Pilomyxoid Astrocytomas From Ependymomas but Shows No Differences in Expression Level in Ependymomas From Infants Versus Older Children or Among Molecular Subgroups.
J Neuropathol Exp Neurol. 2016; 75(4):295-8 [PubMed] Article available free on PMC after 01/04/2017 Related Publications
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miR-15a and miR-24-1 as putative prognostic microRNA signatures for pediatric pilocytic astrocytomas and ependymomas.
Tumour Biol. 2016; 37(7):9887-97 [PubMed] Related Publications
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Pediatric thalamic tumors in the MRI era: a Canadian perspective.
Childs Nerv Syst. 2016; 32(2):269-80 [PubMed] Related Publications
METHODS: We performed a Canadian multicenter retrospective review of pediatric thalamic tumors presenting during the MRI era (1989-2012). Radiology and pathology were reviewed by central independent reviewers. Paraffin shavings for RNA extraction were taken and tested for fusion events involving KIAA1549:BRAF. Tumors were classified as unilateral or bithalamic based on their origin on imaging. Univariate and multivariate analyses on factors influencing survival were performed.
RESULTS: Seventy-two thalamic tumors were identified from 11 institutions. Females represented 53% of the study population, and the mean age at presentation was 8.9 years. Sixty-two tumors were unilateral and 10 bithalamic. Unilateral tumors had a greater propensity to grow inferiorly towards the brainstem. These tumors were predominantly low grade in comparison to bithalamic tumors which were high-grade astrocytomas. The 5-year overall survival was 61 ± 13% for unithalamic tumors compared to 37 ± 32% for bithalamic tumors (p = 0.097). Multivariate analysis indicated tumor grade as the only significant prognostic factor for unithalamic tumors. Six unilateral tumors, all low grade, were BRAF fusion positive.
CONCLUSION: Unilateral and bilateral thalamic tumors behave differently. Surgical resection is an appropriate treatment option in unilateral tumors, most of which are low grade, but outcome is not related to extent of resection (EOR). Bilateral thalamic tumors have a poorer prognosis, but the occasional patient does remarkably well. The efficacy of chemotherapy and radiotherapy has not been clearly demonstrated. Novel therapeutic approaches are required to improve the prognosis for malignant unilateral thalamic tumors and bilateral thalamic tumors.
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Phase I study of 5-fluorouracil in children and young adults with recurrent ependymoma.
Neuro Oncol. 2015; 17(12):1620-7 [PubMed] Article available free on PMC after 01/04/2017 Related Publications
METHODS: Patients 22 years of age or less with recurrent ependymoma were treated with bolus dosage 5-FU weekly for 4 weeks followed by a 2-week rest period, defining one cycle. Patients could continue on therapy for 16 cycles. The starting 5-FU dosage was 500 mg/m(2). Dose-limiting toxicity was determined after one cycle. Patients were initially enrolled according to a rolling-6 design; subsequent dose re-escalation phase was based on a 3 + 3 design.
RESULTS: We treated patients at 400 (n = 6), 500 (n = 15), and 650 (n = 5) mg/m(2), with de-escalation due to toxicity. Twenty-three of twenty-six patients enrolled were evaluable. Five patients experienced grade 4 neutropenia (n = 2: 650 mg/m(2); n = 3: 500 mg/m(2)). One patient experienced grade 3 diarrhea. At 500 mg/m(2), the median 5-FU maximal concentration, AUC0-∞, and alpha half-life were 825 µM, 205 µM × h, and 9.9 min, respectively. Interim analysis revealed an association between hematologic toxicity and prior number of chemotherapeutic regimens (P = .03). The study was amended to re-escalate the dosage in a less heavily pretreated cohort of patients.
CONCLUSIONS: These phase I clinical data provide initial pharmacokinetic parameters to describe i.v. bolus 5-FU disposition in children with recurrent ependymoma. Tumor exposures effective in preclinical testing can be achieved with tolerable bolus dosages in patients. Bolus 5-FU is well tolerated and possesses antitumor activity.
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Anaplastic Ependymoma in a Child With Sickle Cell Anemia: A Case Report Highlighting Treatment Challenges for Young Children With Central Nervous System Tumors and Underlying Vasculopathy.
Pediatr Blood Cancer. 2016; 63(3):547-50 [PubMed] Related Publications
Myxopapillary ependymomas in children: imaging, treatment and outcomes.
J Neurooncol. 2016; 126(1):165-74 [PubMed] Related Publications
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Advances in Management of Pediatric Ependymomas.
Curr Oncol Rep. 2015; 17(10):47 [PubMed] Related Publications
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A rare case of malignant pediatric ectomesenchymoma arising from the cerebrum.
Int J Clin Exp Pathol. 2015; 8(7):8545-50 [PubMed] Article available free on PMC after 01/04/2017 Related Publications
Temozolomide Treatment for Pediatric Refractory Anaplastic Ependymoma with Low MGMT Protein Expression.
Pediatr Blood Cancer. 2016; 63(1):152-5 [PubMed] Related Publications
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The Role of Fast Cell Cycle Analysis in Pediatric Brain Tumors.
Pediatr Neurosurg. 2015; 50(5):257-63 [PubMed] Related Publications
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Imaging Changes in Pediatric Intracranial Ependymoma Patients Treated With Proton Beam Radiation Therapy Compared to Intensity Modulated Radiation Therapy.
Int J Radiat Oncol Biol Phys. 2015; 93(1):54-63 [PubMed] Related Publications
METHODS AND MATERIALS: Seventy-two patients with nonmetastatic intracranial ependymoma who received postoperative RT (37 PBRT, 35 IMRT) were analyzed retrospectively. MRI images were reviewed by 2 neuroradiologists.
RESULTS: Sixteen PBRT patients (43%) developed postradiation MRI changes at 3.8 months (median) with resolution by 6.1 months. Six IMRT patients (17%) developed changes at 5.3 months (median) with 8.3 months to resolution. Mean age at radiation was 4.4 and 6.9 years for PBRT and IMRT, respectively (P = .06). Age at diagnosis (>3 years) and time of radiation (≥3 years) was associated with fewer imaging changes on univariate analysis (odds ratio [OR]: 0.35, P = .048; OR: 0.36, P = .05). PBRT (compared to IMRT) was associated with more frequent imaging changes, both on univariate (OR: 3.68, P = .019) and multivariate (OR: 3.89, P = .024) analyses. Seven (3 IMRT, 4 PBRT) of 22 patients with changes had symptoms requiring intervention. Most patients were treated with steroids; some PBRT patients also received bevacizumab and hyperbaric oxygen therapy. None of the IMRT patients had lasting deficits, but 2 patients died from recurrent disease. Three PBRT patients had persistent neurological deficits, and 1 child died secondarily to complications from radiation necrosis.
CONCLUSIONS: Postradiation MRI changes are more common with PBRT and in patients less than 3 years of age at diagnosis and treatment. It is difficult to predict causes for development of imaging changes that progress to clinical significance. These changes are usually self-limiting, but some require medical intervention, especially those involving the brainstem.
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Methotrexate administration directly into the fourth ventricle in children with malignant fourth ventricular brain tumors: a pilot clinical trial.
J Neurooncol. 2015; 125(1):133-41 [PubMed] Article available free on PMC after 01/04/2017 Related Publications
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Use of proton therapy for re-irradiation in pediatric intracranial ependymoma.
Radiother Oncol. 2015; 116(2):301-8 [PubMed] Related Publications
MATERIALS AND METHODS: Twenty patients underwent 33 PRT re-irradiation courses for recurrent or metastatic lesions between June 2004 and February 2015 at Massachusetts General Hospital.
RESULTS: The majority of patients were female (60%), with infratentorial tumors (90%), anaplastic histology (55%), and initially received 55.8 GyRBE (52.2-59.4) involved field (IF) PRT. First failure was local (55%), distant (30%) or both (15%) at a median time of 23.9 months (9.9-98.5) from first treatment. Salvage therapy included re-resection (75%), chemotherapy (60%) and IFPRT (70%) to a median dose 50.4 GyRBE (35-55.8) in the majority of patients. The median follow-up was 37.8 months (5.5-138.0). Three year OS and PFS are 78.6% (95% CI 67.6-89.6) and 28.1% (95% CI 15.6-40.6), respectively. Longer OS was significantly associated with surgical resection of recurrent disease (HR 9.19, 95% CI 1.27-66.44, p=0.028). The pattern of second failure after re-irradiation was directly related to the pattern of first failure (p<0.01). Three of 14 patients (21.4%) locally re-treated experienced grade 2 radiation-associated treatment change.
CONCLUSIONS: Proton therapy appears safe and efficacious for the re-treatment of recurrent intracranial ependymoma.
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Incidence and survival of childhood central nervous system tumors: A report of the regional registry of childhood cancers in Auvergne-Limousin.
Neurochirurgie. 2015; 61(4):237-43 [PubMed] Related Publications
PATIENTS AND METHODS: We report incidence and survival data for all CNST (International Classification of Diseases for Oncology third edition, category III or Xa) recorded in children under 15 years of age by the Auvergne-Limousin cancer registry for the period 1986-2009.
RESULTS: Annual incidence of all CNST was 3.27 per 100,000 and the male to female ratio was 0.95. Over 45.0% of CNST were glial. Astrocytomas (36.2%) showed the highest incidence for each age group except between 1 and 4 years where embryonal tumors were more common. For all CNST, no significant variation in incidence over time was observed for the evaluated period of 23 years (annual percent change: -0.4%, 95% CI, [-2.8-2.1]). Globally, 5 years overall survival was 67% [59-73] and had increased by more than 16% between 1986-1999 and 2000-2009, mainly due to better survival for astrocytomas, other gliomas, ependymomas and choroid plexus tumors (P=0.01).
CONCLUSION: We report that the incidence of CNST in Auvergne-Limousin is similar to that in the literature and did not increase between 1986 and 2009. In addition, 5 years overall survival increased after 1999, especially for surgically treatable tumors.
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Posterior fossa ependymoma in childhood: 60 years event-free survival after partial resection—a case report.
Childs Nerv Syst. 2015; 31(9):1573-6 [PubMed] Article available free on PMC after 01/04/2017 Related Publications
Long-term life expectancy for children with ependymoma and medulloblastoma.
Pediatr Blood Cancer. 2015; 62(11):1986-91 [PubMed] Related Publications
METHODS: A retrospective analysis was conducted using the SEER Program. Children (ages 0-19 years) from 1973 to 2011 with a diagnosis of MB or IE were identified. A cohort was created of potentially cured patients who survived 5 years from diagnosis. Cox proportional hazards models and Kaplan-Meier estimates were utilized to analyze long-term survival.
RESULTS: We identified 876 patients with MB and 474 patients with IE who were alive 5 years from diagnosis. Patients with MB had a 30-year overall survival (OS) and cancer-specific survival (CSS) of 70.2% and 80.1%, respectively. Patients with IE had a 30-year OS and CSS of 57.3% and 68.8%, respectively. When comparing MB with IE, MB had improved CSS (P = 0.04) and trended toward increased OS (P = 0.10).
CONCLUSIONS: A significant number of deaths due to disease occur for several decades after treatment for both IE and MB. Despite this, the potential for long-term survival exists in 5-year survivors of both histologies. If alive at 5 years from diagnosis, patients with MB tend to have a lower risk of death from disease compared to those with IE.
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Systematic comparison of MRI findings in pediatric ependymoblastoma with ependymoma and CNS primitive neuroectodermal tumor not otherwise specified.
Neuro Oncol. 2015; 17(8):1157-65 [PubMed] Article available free on PMC after 01/04/2017 Related Publications
METHODS: All 66 centrally reviewed cases were obtained from the database of the German multicenter HIT trials. We systematically analyzed the initial MRI scans at diagnosis according to standardized criteria, and paired comparison was performed for EBL and EP, as well as for EBL and CNS-PNET NOS.
RESULTS: We found differences between EBL and EP regarding age at diagnosis, MR signal intensity, tumor margin and surrounding edema, presence and size of cysts, and contrast enhancement pattern. Although MRI appearance of EBL shares many features with CNS-PNET NOS, we revealed significant differences in terms of age at diagnosis, tumor volume and localization, tumor margins, edema, and contrast enhancement.
CONCLUSION: This is the first study that systematically compares multiple parameters of MRI in pediatric EBL with findings in EP and CNS-PNET NOS. Although a definite differentiation by means of MRI alone might not be feasible in the individual case, we identify significant differences between these tumor entities.
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Age-related changes of susceptibility-weighted imaging in subependymal nodules of neonates and children with tuberous sclerosis complex.
Brain Dev. 2015; 37(10):967-73 [PubMed] Related Publications
METHODS: Images of 17 children (age range, 0-14years; mean, 5.5years) with TSC were retrospectively assessed. Changes in signals of SENs on SWI and filtered phase images were scored by two experienced radiologists using a three-point scale. The relation was assessed between patients' age and the average scores of SENs. The phase shift of SENs on the filtered phase image was also measured, and the correlation between age and phase shift was evaluated. Calcification in SENs on CT was compared to the finding on SWI and filtered phase image in four children.
RESULTS: No change in signal was found in neonates in SENs on both SWI and filtered phase image. There was a positive correlation between the average scores of SENs and patients' age on both SWI and filtered phase image (Spearman ρ=0.64, P=0.006; ρ=0.71, P=0.001, respectively). There was a negative correlation between the patients' age and mean phase shift (Spearman ρ=-0.86, P<0.001). Comparable or slight lower detectability of susceptibility changes was revealed in SENs on SWI and filtered phase image, compared to calcification on CT.
CONCLUSIONS: There are age-related changes in susceptibility and phase in SENs, which may reflect the appearance of calcification in children with TSC. No magnetically susceptible changes were detected in neonates, suggesting a lack of calcification.
Intradural Extramedullary Ependymoma with Leptomeningeal Dissemination: The First Case Report in a Child and Literature Review.
World Neurosurg. 2015; 84(3):865.e13-9 [PubMed] Related Publications
CASE DESCRIPTION: We report on an 11-year-old girl presenting with a 1-month history of neck pain, left arm weakness, paresthesia in the fingers of the left hand and gait disturbances. Magnetic resonance imaging on admission revealed an intradural extramedullary cystic lesion at the cervical level with craniospinal leptomeningeal nodules causing mild hydrocephalus. The multicystic lesion was surgically removed and neuropathologic examination revealed a World Health Organization grade II ependymoma. The patient underwent adjuvant radiotherapy with progressive reduction of the metastatic nodules. At her 2-year follow-up, the patient was symptom free with no evidence of recurrence on magnetic resonance imaging.
CONCLUSIONS: Although a rare entity, intradural extramedullary ependymomas should be included in the differential diagnosis of intradural extramedullary lesions in children. Surgical treatment seems to play a pivotal role in the prognosis of these rare tumors, with a possible role for adjunctive radiotherapy in the case of recurrence, anaplastic transformation, and metastasis.
An open-label, two-stage, phase II study of bevacizumab and lapatinib in children with recurrent or refractory ependymoma: a collaborative ependymoma research network study (CERN).
J Neurooncol. 2015; 123(1):85-91 [PubMed] Article available free on PMC after 01/04/2017 Related Publications
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Proton beam therapy for pediatric ependymoma.
Pediatr Int. 2015; 57(4):567-71 [PubMed] Related Publications
METHODS: Proton beam therapy was conducted for six patients (three boys and three girls; age, 2-6 years; median, 5 years) with ependymoma. The tumors were WHO grades 2 and 3 in two and four patients, respectively. All patients underwent surgery (subtotal and gross total resection in three patients each) and proton beam therapy at doses of 50.4-61.2 GyE (median, 56.7 GyE). The mean doses to normal brain tissue in proton beam therapy and photon radiotherapy were simulated using the same treatment planning computed tomography images.
RESULTS: All patients completed the planned irradiation. The follow-up period was 13-44 months (median, 24.5 months) from completion of proton beam therapy and all patients were alive at the end of this period. Local recurrence in the treatment field occurred in one patient at 4 months after proton beam therapy at 50.4 GyE. Alopecia and mild dermatitis occurred in all patients, but there was no severe toxicity. One patient had a once-off seizure after proton beam therapy and alopecia persisted in another patient for 31 months, but no patients had difficulty with daily life. The simulation showed that proton beam therapy reduces the dose to normal brain tissue by approximately half compared with photon radiotherapy.
CONCLUSIONS: Proton beam therapy for pediatric ependymoma is safe, does not have specific toxicities, and can reduce irradiation of normal brain tissue.
Preclinical examination of clofarabine in pediatric ependymoma: intratumoral concentrations insufficient to warrant further study.
Cancer Chemother Pharmacol. 2015; 75(5):897-906 [PubMed] Article available free on PMC after 01/04/2017 Related Publications
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Nanoparticle mediated silencing of DNA repair sensitizes pediatric brain tumor cells to γ-irradiation.
Mol Oncol. 2015; 9(6):1071-80 [PubMed] Article available free on PMC after 01/04/2017 Related Publications
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Severe cyanide poisoning from an alternative medicine treatment with amygdalin and apricot kernels in a 4-year-old child.
Wien Med Wochenschr. 2015; 165(9-10):185-8 [PubMed] Related Publications
PATIENTS AND METHODS: Case report.
RESULTS: We describe a case of severe cyanide poisoning arising from CAM use. A severely agitated, encephalopathic, unresponsive 4-year-old boy (initial Glasgow Coma Scale of 3) with a history of metastatic ependymoma was brought to our emergency department by ambulance services. Initial blood gas analysis demonstrated severe metabolic/lactic acidosis. On detailed questioning of the parents, the use of CAM including intravenous and oral "vitamin B 17" (amygdalin) and oral apricot kernel was reported. After administering sodium thiosulfate, rapid improvement in his medical condition with complete recovery without need for further intensive care treatment was seen. Serum cyanide level was markedly elevated.
CONCLUSIONS: Cyanide poisoning can be the cause of severe encephalopathy in children receiving CAM treatment with substances containing cyanogenic glycosides.
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EZH2 expression is a prognostic factor in childhood intracranial ependymoma: a Canadian Pediatric Brain Tumor Consortium study.
Cancer. 2015; 121(9):1499-507 [PubMed] Related Publications
METHODS: Tissue microarray immunostaining was performed on 180 ependymoma samples from 12 of 16 Canadian pediatric centers. Expression levels of EZH2, Ki-67, B lymphoma Moloney-murine leukemia virus insertion region 1 homolog, tumor protein 16 (P16), Y-box binding protein 1, phosphorylated protein kinase B (pAKT), and epidermal growth factor receptor were evaluated. Cox regression analyses were performed, and the Kaplan-Meier method was used to construct survival curves.
RESULTS: EZH2 expressed in 16% of tumors was associated with inferior 5-year overall survival. Ki-67 and pAKT levels were associated with a poor outcome in patients with posterior fossa ependymoma, and the absence of P16 was associated with a poor outcome in patients with supratentorial ependymoma. Multivariate analysis revealed that younger age and EZH2 expression (95% confidence interval, 1.1-36.0) were independent markers of a poor prognosis.
CONCLUSIONS: EZH2 is a novel, independent marker of a poor prognosis in patients with ependymoma, especially in those who have tumors located in the posterior fossa. EZH2, pAKT, and P16 are potential therapeutic targets, particularly for patients who have tumors in which standard gross total resection plus fractionated radiotherapy is not feasible.
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Prognostic marker analysis in pediatric intracranial ependymomas.
J Neurooncol. 2015; 122(2):255-61 [PubMed] Related Publications
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Treatment of pediatric Grade II spinal ependymomas: a population-based study.
J Neurosurg Pediatr. 2015; 15(3):243-9 [PubMed] Related Publications
METHODS: The Surveillance Epidemiology and End Results (SEER) database was used to analyze subjects younger than 18 years with histologically confirmed diagnoses of Grade II spinal cord ependymoma from the years 1973 to 2008. Descriptive data on the demographic characteristics of this cohort and the associated treatment patterns are shown. The Kaplan-Meier method was used to estimate overall survival at 1, 2, 5, and 10 years.
RESULTS: This cohort comprised 64 pediatric subjects with Grade II spinal ependymoma. The median age was 13 years, nearly half of the patients were male, and most were white (84%). The median follow-up was 9.2 years. Overall survival at 5 and 10 years was 86% and 83%, respectively. Gross-total resection was achieved in 57% of subjects, and radiation therapy was administered to 36%. Radiation therapy was administered to 78% of subjects after subtotal resection but only to 19% of patients after gross-total resection; this difference was significant (p < 0.001). In a multivariate regression model analyzing sex, age at diagnosis, year of diagnosis, radiotherapy, and extent of resection, female sex was found to be an independent predictor of decreased mortality (HR 0.15 [95% CI 0.02-0.94], p = 0.04).
CONCLUSIONS: These data show long-term outcomes for pediatric patients with Grade II spinal ependymoma. Radiotherapy was more likely to be administered in cases of subtotal resection than in cases of gross-total resection. Female sex is associated with decreased mortality, while other demographic or treatment modalities are not.
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The role of resection alone in select children with intracranial ependymoma: the Canadian Pediatric Brain Tumour Consortium experience.
Childs Nerv Syst. 2015; 31(1):57-65 [PubMed] Related Publications
METHODS: We conducted a retrospective review of children with intracranial ependymoma in 12 Canadian centers. Patients who had GTR of their tumor and no upfront radiotherapy were identified. Immunostaining was performed for Ki-67, epidermal growth factor receptor (EGFR), and EZH2 on archived tissue. The Kaplan-Meier survival analysis was performed and compared with those who had GTR followed by radiation.
RESULTS: Twenty-six children were identified treated with GTR alone at diagnosis; 12 posterior fossa ependymoma (PFE) WHO grade II, and 14 supratentorial ependymoma (STE). Progression-free survival (PFS) in ependymoma treated with GTR alone at diagnosis was inferior in those with high Ki-67 or positive EZH2 immunostaining. Survival was inferior for patients less than 2 years old at diagnosis (p = 0.002). Survival was comparable to PFE WHO grade II and STE who had GTR followed by radiation (p = 0.62). Five-year PFS and overall survival (OS) of those treated with GTR alone were 60 and 70% respectively for PFE and 45 and 70% respectively for STE (p = 0.2; 0.55).
CONCLUSIONS: This study suggests that there is a subset of children with certain biologic features who, in the setting of a prospective clinical trial, might be candidates for observation following GTR. Good risk factors for this approach include age of 2 years or older, low Ki-67, and negative EZH2. If relapse occurs, it may be confined to the primary site, allowing for possible salvage with GTR followed by XRT.
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